Cardiogenic Shock - Diagnosis and Treatment

Chapter 16 / Heart Disease in the Child 257
mortality and 85% freedom from reoperation at 5 yr (60). Transvalvular balloon dilata-
tion of critical aortic stenosis, first reported in 1986 (61), is now used in most centers
for initial treatment. It has been shown to be as effective as surgery in terms of gradient
relief and appearance of aortic regurgitation. Egito et al. (62) reported the results of
balloon dilatation of 33 neonates with critical aortic stenosis, performed between 1985
and 1991. Overall mortality was 12%, and midterm results were similar to those of
surgery (88% survival at 8 yr). Femoral pulse loss occurred in 65% of patients. Aortic
valvotomy was reported through the common carotid artery with encouraging results
and few complications (63,64). Balloon dilatation of the aortic valve has been
attempted in a fetus with unsatisfactory results (65).
HYPOPLASTIC LEFT-HEART SYNDROME
Hypoplastic left-heart syndrome is a collective term describing a group of cardiovas-
cular malformations in which there is aortic valve hypoplasia, stenosis, or atresia with
either hypoplasia or absence of the left ventricle and, as a consequence, hypoplasia of
the ascending aorta. The mitral valve usually is stenotic or atretic (66). The patient usu-
ally presents when the ductus arteriosus closes. Occasionally, patients with this disease
can present soon after birth, when the interatrial communication is obstructive. As the
ductus arteriosus closes, systemic and coronary flow are compromised and the baby
becomes dusky, tachypneic, and acidotic. This can happen in the first 24–48 h or later.
If not treated, cardiogenic shock will ensue and death will follow in a few hours.
The diagnosis is made by two-dimensional echocardiogram; cardiac catheterization
is not required routinely. The approach to the newborn with the syndrome historically
has been to offer the parents one of three options: (1) do nothing; (2) do a three-stage
palliative operation (Norwood procedure); or (3) do a cardiac transplantation. In recent
years, because of improved results of the latter two options, many physicians feel that
the first option is no longer ethical.
When a surgical approach is elected, prostaglandin E
1
is started and an arterial line
should be placed. Because the pulmonary circulation is in series with the systemic cir-
culation while the latter is supplied by the ductus arteriosus, decrease of the pulmonary
resistance causes pulmonary overcirculation and may compromise the systemic circu-
lation. Thus, maneuvers to increase pulmonary resistance may be needed. These
include hypoventilation or the addition of 2–4% CO
2
to the ventilation mixture.
Inotropic drugs may not be necessary or beneficial (66).
Comparable survival data are reported for the Norwood procedure and cardiac trans-
plantation. Hehrlein et al. (67) reported better quality of life for patients who had car-
diac transplantation. Kern et al. (68) reported 67 patients with hypoplastic left-heart
syndrome, of whom 47 had the Norwood procedure between 1990 and 1996. Survival
was 77% of the surgically treated patients, 61% when including those who did not
undergo the operation. The 5-yr actuarial survival for patients who underwent opera-
tions was 61%. Others report similar results (69). Jenkins et al. reported the 1-yr sur-
vival of 231 patients operated at 4 centers between 1989 and 1994. Patients who were
intended for transplantation had 61% 1-yr survival, whereas those intended for staged
procedure had 42% 1-yr survival (70). Somewhat better survival (88%) has been
reported in recent years for patients who had a cardiac transplantation. The long-term
prognosis is uncertain for both procedures. Mental development is a concern in this
patient group (71); Mahle et al. (72) reviewed school-age survivors and found 18%
were mentally retarded and 30% required special education.
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